Cardiomiopatias: conceito e classificação / Definition and classification of cardiomyopathies

Cardiomiopatias compõem um grupo heterogêneo de doenças do músculo cardíaco vinculadas a múltiplas etiologias e uma variedade de expressões fenotípicas. Em 1995, a Organização Mundial de Saúde as definiu como sendo "doenças do miocárdio associadas com disfunção cardíacas", e classificadas em: dilatada, hipertrófica, restritiva e cardiomiopatia arritmogênica do ventrículo direito. Um comitê de especialistas da American Heart Association (AHA) em 2006 conceituou que as cardiomiopatias compreendem um "grupo heterogêneo de doenças do miocárdio associadas com disfunção mecânica e/ou elétrica, e que usualmente (mas não invariavelmente) exibem dilatação ou hipertrofia ventriculares inapropriadas", e são devido a uma variedade de causas, frequentemente genéticas. Foram divididas em 2 tipos: primárias e secundárias (Tabela 1). Foi incluída ainda uma importante subcaracterização das cardiomiopatias primárias, dividida em 3 grupos principais: genético, misto e adquirido (Tabela 2). Em 2007, um posicionamento do comitê europeu mudou a conceituação: uma afecção miocárdica na qual o músculo cardíaco é funcionalmente e estruturalmente anormal, em ausência de doença coronariana, hipertensão arterial, doença valvular, defeito cardíaco congênito, suficiente para causar a anormalidade miocárdica observada...

The cardiomyopathies are an important and complex group of heart muscle diseases with multiple tiologies and heterogeneous phenotypic expression. The WHO classification published in 1995 proposed “ Cardiomyopathies are defined as diseases of the myocardium associated with cardiac dysfunction”, and they are classified as dilated, hypertrophic, restrictive, and arrhythmogenic right ventricular cardiomyopathy. In 2006 AHA scientific statement, the original distinction into primary and secondary forms was reintroduced. A sub classification of cardiomyopathies into familial/genetic and non-familial/non-genetic was considered of help in orienting towards genetic mutational analysis and creening. All this was changed in 2007 by ESC, when they clearly stated that cardiomyopathy is ‘a myocardial disorder in which heart muscle is structurally and functionally abnormal in the absence of coronary artery disease, hypertension, valvular disease, and congenital heart diseases’. While accepting and reinforcing the idea advanced by the AHA statement to separated cardiomyopathies into familial/genetic and non-familial/non-genetic, the traditional separation of primary and secondary (specific) cardiomyopathies was abolished...

[Different expressions of inflammatory cytokines in two types of cardiac hypertrophy in rats].

OBJECTIVE: To compare the different expressions of cardiac inflammatory cytokines including tumor necrosis factor-alpha (TNF-alpha), interleukin-1beta(IL-1beta), interleukin-6 (IL-6) in two types of cardiac hypertrophy rat models induced by volume overload and pressure overload. METHODS: Volume overload-induced cardiac hypertrophy was established by abdominal arteriovenous fistula (ACF) and pressure overload-induced cardiac hypertrophy was developed by constriction of aorta (CA). Heart weight measurement and histological examination were performed 1 week or 2 weeks after the operation respectively. The cytokine expression was measured by enzyme linked immunosorbent assay. RESULTS: All the operated groups developed cardiac hypertrophy. The left ventricular fractional shortening of each operated group had no significant difference with the sham-operated groups respectively. As far as the total amount of each cytokine in left ventricular myocardium was concerned, compared to the sham-operated groups, IL-6 and IL-1beta both increased significantly in CA groups [IL-6(23 722+/-8 671)pg vs (17 693+/-5 705)pg,P<0.05 ;IL-1beta(335+/-95)pg vs (159+/-99) pg,P<0.01].There was no difference of TNF-alpha between operated and sham-operated groups in ACF or CA groups . CONCLUSION: The contents of IL-6 and IL-1beta in myocardium increased in pressure overload-induced cardiac hypertrophy.

Cardiac hypertrophy: mechanisms and therapeutic opportunities.

Cardiac hypertrophy and heart failure are major causes of morbidity and mortality in Western societies. Many factors have been implicated in cardiac remodeling, including alterations in gene expression in myocytes, cardiomyocytes apoptosis, cytokines and growth factors that influence cardiac dynamics, and deficits in energy metabolism as well as alterations in cardiac extracellular matrix composition. Many therapeutic means have been shown to prevent or reverse cardiac hypertrophy. New concepts for characterizing the pathophysiology of cardiac hypertrophy have been drawn from various aspects, including medical therapy and gene therapy, or use of stem cells for tissue regeneration. In this review, we focus on various types of cardiac hypertrophy, defining the causes of hypertrophy, describing available animal models of hypertrophy, discussing the mechanisms for development of hypertrophy and its transition to heart failure, and presenting the potential use of novel promising therapeutic strategies derived from new advances in basic scientific research.

Competition for intrathoracic space reduces lung capacity in patients with chronic heart failure: a radiographic study.

BACKGROUND: The purpose of this study was to determine the influence of changes in cardiac size on total lung volume in patients with chronic heart failure compared to control subjects. METHODS: Forty-four patients and age-, gender-, and height-matched control participants were recruited. All participants underwent posteroanterior and lateral chest radiography for volumetric estimations of the total thoracic cavity (TTC), diaphragm, heart, and lungs. To assess the relationship between chronic heart failure severity and cardiac enlargement, patients with chronic heart failure were classified into groups based on New York Heart Association class, as follows: class I and II, n = 26 (group A); class III and IV, n = 18 (group B). RESULTS: There was no difference between the groups for TTC volume (TTCV) [p = 0.56]. Cardiac volumes were significantly different between all groups for both the absolute volumes (p < 0.001) were calculated as a percentage of TTCV (p < 0.001), with the largest cardiac volumes in group B (twice the volume of healthy control subjects). When expressed as a percentage of TTCV, there also was a clear reduction in lung volumes as a function of disease severity (p < 0.001). CONCLUSIONS: The present study demonstrates a close relationship between the severity of heart failure and cardiac size. These changes in cardiac size within a closed thoracic cavity may pose significant constraints on the lungs, resulting in reductions in lung volumes that likely play a major role in the restrictive breathing patterns often reported in patients with chronic heart failure.

Usefulness of stanford scale of intimal hyperplasia assessed by intravascular ultrasound to predict time of onset and severity of cardiac allograft vasculopathy.

BACKGROUND: The purpose of this study was to assess the prognostic value of a single IVUS result described by the Stanford scale to predict CAV development. METHODS: Inclusion criteria were heart transplantation (OHT) before 1997 and at least one IVUS performed before 1998. IVUS studies were performed in 37 patients at 37 +/- 26 months after OHT. Based on the Stanford scale, were divided patients into Four groups: group I (grade 0 or 1): n = 4, 42 +/- 19 years, 2 men/2 women; group II (grade 2): n = 10, 44 +/- 15 years, 9 men/1 woman; group III (grade 3): n = 11, 48 +/- 11 years, 11 men; and group IV (grade 4): n = 12, 46 +/- 8 years, 12 men. We compared the incidence and time of onset of clinically significant CAV, namely significant coronary lesions, myocardial infarction and death caused by CAV. RESULTS: There was no CAV diagnosed in group I. The rates of CAV in coronary angiograms in groups II, III and IV were: 80%, 36%, and 75%, respectively. Significant CAV was found in 30%, 9%, and 50% of patients, respectively. Average times of onset of any CAV in groups II, III and IV were 4.9, 5.6, and 3.3 years, and for significant CAV were 4.1, 3.6, and 5.5 years, respectively. Deaths in groups I to IV were 1, 4, 2, and 5, respectively. CAV was the reason for death in 1 patient from group III, and 3 patients from group IV. CONCLUSIONS: Extreme grades on the Stanford scale (0, 1, and 4) describing a single IVUS study in OHT recipients appear useful to stratify patients with the lowest versus the highest risk of CAV development.

[Clinical and morphologic classification of valve replacement with giant heart].

From Apr. 1977 to Dec. 1992, 285 patients with giant heart (CTR > 0.70) underwent valve replacement. According to the index of cardiac chamber enlargement, the patients were clinically and morphologically classified in to four types. Type of left atrium (153 cases, 56.3%) is most common and has good surgical result. The operative mortality was reduced from 11.1% to 1.3%. Type of left ventricle (42, 15.2%) was high risk for surgical treatment, the operative mortality was 33.3%. Type of right cardiac enlargement (17 cases) is less common than other types, and is mainly for tricuspid valve incompetence. Type of total cardiac enlargement was found in 60 cases (22.1%), it was conventionally complicated with the disfunction of other organs with a higher operative mortality of 23.3%. We conclude that this classification represents the degree of cardiac chamber enlargement and the severity of heart disfunction, and has good coincidence with surgical result. It is helpful to identifying operative candidate for valve replacement with giant heart.

[Pathological classification and clinical implications on rheumatic heart disease with giant heart].

Between Jan. 1982 and July, 1993, 102 patients with rheumatic giant heart disease (cardio-thoracic ratio > or = 0.8) underwent valve replacement. Heart functional class III was in 54 patients, and class IV in 48. Early mortality was 8.8%, late mortality 3.6%. We classified the patients into five types, based on the valvular lesions, dilated degree of each atrial and ventricular chamber: Type I, giant left atrium (LA); Type II, obvious enlargement of LA and left ventricle (LV); Type III; obvious enlargement of LA, right atrium and ventricule (RA and RV); Type IV; all chambers obviously dilated; Type V, giant LA and RA. Clinical results showed that this kind of classification can reflect kinds of diseased valves, pathophysiological changes and clinical characteristics, and postoperative early and late outcomes, also can offer useful suggestions to operative method selection, and has great clinical significance for perioperative management.

Classification of hypertensive cardiomyopathy.

A clinical pathophysiological classification of hypertensive cardiomyopathy has been established on the basis of the degree to which the heart is affected by chronic, systemic arterial hypertension: Degree I: Asymptomatic patients without left ventricular hypertrophy but with left ventricular diastolic dysfunction according to Doppler mitral inversion relation (E/A < 0.9) or to gamma scintigraphy (peak filling rate reduction < or = 2.7 EDC.s-1. These patients are classified as Group 1. Degree II: Asymptomatic or mildly symptomatic patients (New York Heart Association class I) with echocardiographic left ventricular hypertrophy; classified as Group IIA or IIB according to whether weight-adjusted maximal oxygen uptake is normal or below normal, respectively. Degree III: The basic characteristic is the presence of congestive heart failure with normal ejection fraction (EF > or = 50%). Two subsets can be distinguished on the basis of degree of hypertrophy: Group IIIA, with a mass/volume index > 1.8, and IIIB with a mass/volume index < 1.8. The differences between the two are as follows: patients classified as IIIA had a lower rate of regional ischaemia, a higher ejection fraction, a more frequently audible fourth sound, rarely a third sound and a cardiothoracic ratio < 0.5; IIIB patients had a higher prevalence of regional ischaemia (thallium-positive), a frequently audible third sound and a cardiothoracic ratio > 0.5. Degree IV: This category is characterized by the presence of depressed contractility, which could cause heart failure, by an ejection fraction < 50% and an increase in ventricular volumes. Echocardiography shows increased distance between mitral point E and the septum.

Long-term echocardiographic follow-up of acromegalic heart disease.

Heart muscle disease in acromegaly manifests usually as cardiac hypertrophy. Based on a retrospective analysis, it was suggested that cardiac hypertrophy is slowly reversible after normalization of plasma growth hormone levels. The reversibility of acromegalic heart muscle disease during and after treatment of acromegaly was studied prospectively. A cohort of 78 patients was examined echocardiographically in 1981, and 38 survivors of this group were reexamined 10 years later. Patients were classified according to original hormonal activity in 1981, and change in hormonal activity during follow-up into the following 4 groups: group I--hormonally inactive for entire follow-up (n = 10); group II--hormonally active for entire follow-up (n = 11); group III--initially hormonally inactive with later resurgence (n = 6); and group IV--initially hormonally active with later normalization of growth hormone levels (n = 11). No significant echocardiographic changes occurred during follow-up in group I. Left ventricular posterior wall and septal diastolic thickness, and left ventricular mass increased significantly (all p < 0.05) in group II. Left ventricular posterior wall thickness, mass and diastolic volume increased significantly (p < 0.05, < 0.01 and < 0.001, respectively) in group III. On the contrary, there were significant decreases in left ventricular mass, and both diastolic and systolic left ventricular volumes (p < 0.01, < 0.05 and < 0.05, respectively) in group IV. It is concluded that both hypertrophy and dilatation of the left ventricle in acromegaly are slowly reversible after successful treatment. On the contrary, continuing or relapsed hyperproduction of growth hormone causes further deterioration of acromegalic heart disease.

A new classification of left ventricular geometry in patients with cardiac disease based on M-mode echocardiography.

M-mode echocardiograms of 202 cardiac patients were studied with respect to the pattern of left ventricular (LV) geometry. Patients with normal LV mass and volume were separated from those who had LV hypertrophy or enlargement on the basis of LV mass and volume indexed to body surface area. The relative wall thickness that is currently used to classify LV hypertrophy/enlargement was found to be inadequate for differentiating between concentric and eccentric types of LV hypertrophy. A new M-mode echocardiographic classification is therefore proposed that accurately separates the different types of LV enlargement; it also allows identification of patients who have chronically dilated left ventricles at the expense of thin walls and thus have normal LV mass.

Radiologic changes in chronic heart failure.

The relationship between chest X-ray findings and NYHA classification or haemodynamic parameters (ejection fraction, end-diastolic and end-systolic volumes by echocardiography, right atrial pressure--RAP, pulmonary artery pressure--PAP and pulmonary wedge pressure--PAWP by right heart catheterization) was evaluated in 44 and 22 patients with chronic congestive heart failure, respectively. In chest X-ray, the cardiothoracic index (CTI) and authors' own classification of pulmonary congestion (PCG) of classes 0, 1 and 2 were used. A significant correlation was found between CTI and NYHA classification or haemodynamic parameters, and between PCG (classes 0 and 2) and echocardiographic parameters or right heart pressures (EDV, ESV, RAP, PAP, PAWP), but there was no significant relationship between X-ray and left ventricular ejection fraction. For a PAWP higher than 18 mmHg and PCG class 2, the sensitivity of X-ray was 86% and specificity 80%. The authors conclude that chest X-ray provides reliable information about the haemodynamic parametres, comparable to echocardiographic and catheterization data.

The diagnostic performance of computer programs for the interpretation of electrocardiograms.

BACKGROUND: Computer programs for the interpretation of electrocardiograms (ECGs) are now widely used. However, a systematic assessment of various computer programs for the interpretation of ECGs has not been performed. METHODS: We undertook a large international study to compare the performance of nine electrocardiographic computer programs with that of eight cardiologists in interpreting ECGs in 1220 clinically validated cases of various cardiac disorders. ECGs from the following groups were included in the sample: control patients (n = 382); patients with left ventricular hypertrophy (n = 183), right ventricular hypertrophy (n = 55), or biventricular hypertrophy (n = 53); patients with anterior myocardial infarction (n = 170), inferior myocardial infarction (n = 273), or combined myocardial infarction (n = 73); and patients with combined infarction and hypertrophy (n = 31). The interpretations of the computer programs and the cardiologists were compared with the clinical diagnoses made independently of the ECGs, and the computer interpretations were compared with those of the cardiologists. RESULTS: The percentage of ECGs correctly classified by the computer programs (median, 91.3 percent) was lower than that of the cardiologists (median, 96.0 percent; P less than 0.01). The median sensitivity of the computer programs was also significantly lower than that of the cardiologists in diagnosing left ventricular hypertrophy (56.6 percent vs. 63.9 percent, P less than 0.02), right ventricular hypertrophy (31.8 percent vs. 46.6 percent, P less than 0.01), anterior myocardial infarction (77.1 percent vs. 84.9 percent, P less than 0.001), and inferior myocardial infarction (58.8 percent vs. 71.7 percent, P less than 0.0001). The median total accuracy level (the percentage of correct classifications) was 6.6 percent lower for the computer programs (69.7 percent) than for the cardiologists (76.3 percent; P less than 0.001). However, the performance of the best programs nearly matched that of the most accurate cardiologists. CONCLUSIONS: Our study shows that some but not all computer programs for the interpretation of ECGs perform almost as well as cardiologists in identifying seven major cardiac disorders.

Preoperative and intraoperative predictors of inotropic support and long-term outcome in patients having coronary artery bypass grafting.

The prognostic value of preoperative symptoms, preoperative left ventricular function, and intraoperative factors as related to postoperative outcome in coronary artery bypass grafting is unclear. This study was performed to identify risk factors that could be used as markers to predict immediate and long-term outcome, knowledge of which might allow physicians to modify these factors to decrease the likelihood of an adverse outcome. We retrospectively evaluated preoperative factors (including age, sex, New York Heart Association [NYHA] classification of symptoms, ejection fraction [EF], wall motion abnormalities, baseline left ventricular end-diastolic pressure [LVEDP], postradiographic contrast injection LVEDP, change in LVEDP with contrast injection, cardiac enlargement, and collateral vessels) and intraoperative factors (duration of bypass and aortic cross-clamp time) in 128 patients. The need for inotropic drug support was used as a marker of immediate outcome. A 36-mo follow-up used death and the postoperative NYHA classification of symptoms as markers of long-term outcome. The various factors associated with the use of inotropes and immediate outcome were analyzed by logistic regression. The factors related to inotrope use (and presumed adverse short-term outcome) in order of decreasing significance were lower EF, older age, cardiac enlargement, female sex, and higher baseline and postcontrast LVEDP. Patients with EF greater than or equal to 55%, but also having wall motion abnormalities and LVEDP change greater than or equal to 10 mm Hg, and all patients with EF less than 55% were more likely to require inotropic drug stimulation after cardiopulmonary bypass. Neither the change in LVEDP nor the presence of wall motion abnormalities independently predicted the need for postoperative inotropic support.(ABSTRACT TRUNCATED AT 250 WORDS)

[Atypical forms of hypertrophic cardiomyopathy]. / Nietypowe postaci kardiomiopatii przerostowej.

UNLABELLED: Hypertrophic cardiomyopathy is characterized by a diverse clinical and morphological spectrum. In this report we describe five patients with atypical hypertrophic cardiomyopathy and significantly dilated atria. Diagnostic difficulties are stressed. All patients underwent clinical examination, noninvasive studies including 2-D echocardiogram, cardiac catheterization. In three patients endomyocardial biopsy was taken and examined by light microscopy. All but one patient were men, ranging in age from 28 years to 40 years at initial examination. In all patients disproportion between mildly elevated or, in two cases normal filling pressures and degree of dilatation of both atria was found. Two patients C.G. and D.T. had auscultatory and roentgenographic findings of mitral stenosis. Following echocardiographic and angiocardiographic studies an increase in left and right ventricular thickness associated with dilatation of both atria was found. Endomyocardial biopsy in both cases did not show endocardial thickening or infiltrative changes. In family S, two patients W.S. and Je.S had marginal left ventricular hypertrophy. The presence of left ventricular hypertrophy in patient Ja. S and finding, after family studies, hypertrophic, obstructive cardiomyopathy in patient A.S. enabled establishing the correct diagnosis. Beside varying degree of hypertrophy in family S, mild dilation of the right ventricle and incomplete right bundle branch block were found. In two patients of family S. restrictive cardiomyopathy was found, in patient Ja. S. during cardiac catheterization at initial presentation, in patient W.S. on doppler transmitral flow velocity examination at late follow-up. Paroxysmal atrial fibrillation was the first symptom in four patients, thromboembolic event in one patient. In four patients pacemaker requirement was found. During long-term follow-up (mean 4.8 years) slowly progressive heart failure associated with further dilatation of atria is observed. Mild doses of diuretics are effective in controlling congestive symptoms. CONCLUSION: in hypertrophic cardiomyopathy significantly dilated atria and clinical signs of mitral stenosis can be present. The presence of myocardial hypertrophy is not necessary to diagnose hypertrophic cardiomyopathy. Familial studies can be helpful in establishing the correct diagnosis.

Diastolic properties of hypertrophied hearts in essential hypertension: classification by left ventricular wall stress.

Hypertensive cardiac hypertrophy of 20 patients was classified as inappropriate hypertrophy (HH-I) and appropriate hypertrophy (HH-II) according to their end-systolic wall stress, as measured by echocardiography. The differences in systolic and diastolic performances among the HH-I and HH-II subjects and 10 normal controls (NC) before and during isoproterenol infusion were investigated. Eight patients had subnormal end-systolic wall stress (inappropriate hypertrophy) and 12, normal end-systolic wall stress (appropriate hypertrophy). Before isoproterenol infusion, normalized peak rate of a change in left ventricular diameter during systole was significantly greater in HH-I (3.5 +/- 0.8/s) than in NC (2.3 +/- 0.5/s) and HH-II (2.6 +/- 0.6/s) (p less than 0.01 and p less than 0.005), but there was no significant difference between HH-II and NC. There was no significant difference in normalized peak rate of a change of left ventricular diameter during the rapid filling phase among the three groups (4.5 +/- 1.2/s in HH-I, 4.0 +/- 1.6/s in HH-II, and 4.2 +/- 0.8/s in NC). During isoproterenol infusion, normalized peak rate of a change of left ventricular diameter during systole was significantly greater in HH-I (7.0 +/- 1.9/s) than in HH-II (4.8 +/- 1.7/s) and NC (4.8 +/- 0.8/s) (p less than 0.05 and p less than 0.01, respectively), but there was no significant difference between HH-II and NC. Normalized peak rate of a change of left ventricular diameter during rapid filling was significantly less in HH-II (4.8 +/- 1.7/s) than in HH-I (7.3 +/- 1.3/s) and NC (6.5 +/- 0.8/s) (p less than 0.005 and p less than 0.005, respectively), but there was no significant difference between HH-I and NC. These results suggest that hypertensive patients with inappropriate hypertrophy have relatively diminished diastolic velocity (supernormal systolic velocity and normal diastolic velocity) before and during isoproterenol infusion, and that hypertensive patients with appropriate hypertrophy have absolutely diminished diastolic velocity during isoproterenol infusion, in spite of normal diastolic velocity before the infusion.